[Cherubism: diagnosis and treatment in the pediatric age]

Area of Science:

• Genetics
• Pediatric Dentistry
• Oral and Maxillofacial Surgery

Background:

• Cherubism is a rare, autosomal dominant genetic disorder characterized by benign fibrous dysplasia of the jawbones.
• It typically presents in early childhood with painless, symmetrical enlargement of the maxilla and mandible, often resolving spontaneously after puberty.

Observation:

• This report details an aggressive case of cherubism with extensive facial bone involvement, significantly impacting facial contours and occlusion.
• The condition was monitored from early childhood through puberty, presenting unique management challenges due to its progressive nature.

Findings:

• Diagnosis relies on clinical, radiological, and histological findings, confirming the replacement of normal bone with cellular fibrous tissue.
• The aggressive nature of this case necessitated multiple conservative surgical interventions to mitigate aesthetic and functional disturbances.

Implications:

• Managing aggressive cherubism requires a careful, individualized approach, balancing surgical intervention with the child's growth and development.
• This case underscores the complexities in determining optimal timing and surgical techniques for cherubism to address functional and emotional well-being.